Salah satu penyumbang kematian terbesar bayi baru lahir adalah kelainan bawaan (congenital anomaly), baik yang disebabkan oleh faktor genetik maupun multifaktorial. Penanganan kelainan bawaan/kongenital secara komprehensif perlu dilakukan untuk menurunkan angka morbiditas dan mortalitasnya. Untuk mencapai penanganan komprehensif, diperlukan pemahaman kelainan tersebut sejak perkembangan embrio (developmental biology) dan bagaimana menegakkan diagnosisnya lebih dini. Sampai saat ini, salah satu penyumbang keterlambatan diagnosis kelainan kongenital adalah keterbatasan alat diagnostik. Selain itu beberapa alat diagnostik masih bersifat invasif dan menggunakan sinar radiasi, sehingga diperlukan alat diagnostik alternatif yang bersifat akurat dan affordable. Genetik molekular merupakan pendekatan diagnostik yang bersifat robust, tidak invasive, tidak memerlukan sinar radiasi, akurat dan affordable.
Oleh karena itu, Fakultas Kedokteran, Kesehatan Masyarakat dan Keperawatan UGM menyelenggarakan The 3rd ISCADB 2019, dengan tema "Current Concept and Management of Congenital Anomaly – From Bench to Bedside and Community”, sebagai ajang diskusi mengenai perkembangan penanganan kelainan kongenital (congenital anomaly), mulai aspek perkembangan saat embrio (developmental biology), prenatal diagnosis, penegakan diagnosis dengan pendekatan genetika molekular dan manajemen terkini berbasis berbasis karakter spesifik pasien (personalized medicine), serta meningkatkan jumlah dan kualitas publikasi peneliti Indonesia yang dicapai dalam konferensi ini.
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CHARACTERISTICS OF CHOLEDOCAL CYST PATIENTS IN PEDIATRIC SURGERY DIVISION HASAN SADIKIN HOSPITAL
CHARACTERISTICS OF CHOLEDOCAL CYST PATIENTS IN PEDIATRIC SURGERY DIVISION HASAN SADIKIN HOSPITAL The 3rd International Symposium on Congenital Anomaly and Developmental Biology Yogyakarta, 8 – 9 August 2019 Presented by : Rieza Nurdinsyah Hadikusumah, dr FACULTY OF MEDICINE PADJADJARAN UNIVERSITY HASAN SADIKIN HOSPITAL BANDUNG 2018 CHARACTERISTICS OF CHOLEDOCAL CYST PATIENTS IN PEDIATRIC SURGERY DIVISION HASAN SADIKIN HOSPITAL Rieza Nurdinsyah N, Emiliana Lia, Rizki Diposarosa Pediatric Surgery Division, Department of Surgery, Hasan Sadikin Hospital Padjadjaran University, Bandung Indonesia _____________________________________________________________________ ABSTRACT Introduction: Choledocal cyst is a cystic dilation of the bile duct, both intra and extra hepatic. It is one of the common cause of obstructive jaundice in children. The prevalence of choledocal cysts are more common in Asian countries, ranging from about 1 in 1000 cases in Japan. The purpose of this study is to describe the characteristic of choledocal cyst patients in Pediatrics Surgery Division Hasan Sadikin Hospital Bandung. Method: This was a descriptive study. Data was obtained from choledocal cyst medical record patients who admitted in pediatric surgical ward in the period January 2014-December 2017. The collected data consist of gender, age, clinical symptoms, diagnostic imaging, and surgical finding. Results: There were 15 patients diagnosed as choledocal cyst, consisting of 12 girls (80%) and 3 boys (12%). Eight patients were in age group > 5 years old (53.3%), 3 patients in group of 2 – 5 years old (20%), there were 2 patients each in group 1 – 2 years old (13,3%) and 0 – 12 month old (13,3%). Jaundice was the most clinical symptoms found in all patients. Abdominal pain was found in 12 patients (80%), abdominal mass in 9 patients (60%), acholic stools and dark urine were found in 7 patients (46.6%), fever found in 6 patients (40%), and other was nausea and vomiting were found in 3 patients (20%), and anorexia was found in one patients (8.3%). The laboratory results showed signs of bile obstruction with increasing in total and direct bilirubin levels and also increasing in gamma GT and alkaline phosphatase in 5 patients (33.3%). Imaging results using ultrasound revealed 5 patients with choledocal cyst type IV (33.3%), type I in 4 patients (26.6%) and type II in 3 patients (20%). MRI was performed in 8 patients and CT Scan was performed in 5 patient. Eleven patients underwent laparotomy exploration and cyst excision and Roux n Y Hepaticojejunostomy with liver Biopsy. Conclusions: Choledocal cyst predominantly found in girls, with age group > 5 years old, and jaundice was the most common symptom. Ultrasound, MRI, and CT are imaging modalities use to diagnose choledocal cyst. Laparotomy exploration, cyst excision and roux and y hepaticojejunostomy are safe surgical procedure to manage choledocal cyst. Keywords: Choledocal cyst, obstructive jaundice, characteristics. Introduction Choledochal cysts are a cystic dilatation of the biliary tree, intra or extrahepatic, causing a biliary obstruction and progressive biliary obstruction, with clinical symptoms such as jaundice, pain, and fever. Choledochal cyst generally related to complications in biliary tract and pancreas. Choledochal cyst cases were relatively seldom found in the western countries, only 1 in 100.000-150.000 cases up to 1 case in 2 million living births. Meanwhile, choledochal cyst prevalence in Asian countries was higher with 33-50% cases reported in Japan reaching 1 case in 1000 person population.1 Choledochal cysts occurred more in females rather than males, with the ratio of 3:1 until 4:1. This case could be found in all ages, but almost 67% cases with an appropriate symptoms were found before reaching 10 years old.1The aim of this study is to describe the caracteristics of choledocal cyst patient in Hasan Sadikin Hospital Bandung. This study use as one of the early data reference to conduct early prevention towards choledochal cysts. Methods This was a descriptive study with a retrospective approach using medical records to obtain the characteristics of choledochal cyst patients in pediatric surgery division of Dr. Hasan Sadikin General Hospital, Bandung in 3 years period. Results The data collection for this study, was from January 2014 – December 2017. Of 15 patients of choledochal cyst hospitalized in pediatric surgery division of Hasan Sadikin Hospital Bandung. The table below (tabel.1) showed demographic data. Table 1. Demographic data Sex : Total % Male Female 3 12 20 80 Age : Total % 0 – 12 months old 1 – 2 years old 2 – 5 years old >5 years old 2 2 3 8 13.3 13.3 20 53.3 Majority subjects was female, with commonly were age of more than 5 years old (53.3%). Table 2. Clinical Symptoms Clinical Symptoms : Total % Jaundice Abdominal Pain Palpable intraabdominal mass Acholic stool Dark colored urine Anorexia Fever Nausea and vomiting 12 15 9 7 7 2 6 3 80 100 60 46.6 46.6 13.3 40 20 The clinical symptoms was obtained from history taking data in medical records. All patients came with abdominal pain. 12 patients with jaundice (80%), 9 patients with palpable intraabdominal mass (60%), 7 patients with pale colored stool and dark colored urine (46.6%), 6 patients with fever (40%), 3 patients with nausea and vomiting (20%), and 2 patients with anorexia (13.3%). Table 3. Laboratory results Parameters : Total % Increased total dan direct bilirubin, Alkali phosphatase dan Gamma GT Increased total dan direct bilirubin, normal Alkali fosfatase dan Gamma GT Increased Alkali phosphatase, normal total dan direct bilirubin 5 3 3 33.3 20 20 The laboratory results revealed the total and direct bilirubin level, alkali phosphatase, and gamma gt level as the information information of the obstruction of bile duct. There were 5 patients with increasing total and direct bilirubin level and alkali phosphatase and gamma gt (33.3%), 3 patients with increasing total and direct bilirubin level and normal alkali phosphatase and gamma gt (20%). 3 patients with increasing alkali phosphatase and gamma gt with normal total and direct bilirubin level (20%). Table 4. Imaging Results Cyst types Total % USG Type 1 Type 2 Type 4 4 3 5 26.6 20 33.3 MRI Type 4 8 100 CT Scan Type 4 5 100 Ultrasonography gives information about the type of choledochal cyst. There were 4 patients with type 1 cyst (26.6%), 3 patients with type 2 cyst (20%), and 5 patients with type 4 cyst (33.3%). MRI and Abdominal CT Scan gives an information more specifically about the type of choledochal cyst. There were 8 patients conducted an MRI and 5 patients conducted an Abdominal CT Scan, from those 8 patiens with an MRI result, 100% showed a type 4 cyst, and from those 5 patients with an abdominal CT Scan result, 100% showed a type 4 cyst. Table 5. Intraoperative Findings Cyst Size : Total % <10 cm >10 cm 8 3 73 20 Operative is a definitive therapy for a choledochal cyst, an intraoperative findings gave information about the type of choledochal cyst. There were 11 patients underwent explorative laparotomy surgery + cyst excision + Roux n Y hepaticojejunostomi + liver biopsy, and 4 patients did not undergo any operative measure. From those 8 patients that underwent surgery, cyst size was found and as many as 8 patients with >10 cm size (73%) and 3 patients with <10 cm size (20%). Discussion Choledochal cysts are a cystic dilatation of the biliary tree, intrahepatic or extrahepatic, causing a biliary obstruction and progressive biliary obstruction.2 Choledochal cyst mostly manifested in the first decade of life. In infants, with age range from 1 to 3 months old, the most frequent symptoms were obstructive jaundice, acholic stool, and hepatomegaly. Clinical appearance of this group could not be distinguished with biliary atresia. Sometimes, a liver fibrosis could followed.4 In older children, a classic triad usually manifested that were acute abdomen, palpable mass, and jaundice. Because the obstruction in this group usually partial, the symptoms were intermittent.2 Laboratory examination showed abnormality caused by biliary tract obstruction, particularly an increased alkali phosphatase level.1 Ultrasonography could help evaluate patients with intraabdominal mass. In this study, from 15 patients of choledochal cyst, there were a majority of females with female:male ratio of 4:1. The highest number of age group was more than 5 years old, there 8 patients, followed by 2-5 years old age group with 3 patients, 1-2 years old age group with 2 patients, and 0-12 months old age group with 2 patients, a late definitive diagnosis of choledochal cyst could cause a complication and later interfere healing process of the patient, this could be caused by lack of socialization and education towards mothers about choledochal cyst. Abdominal pain was the highest number of clinical symptom found in this study followed by jaundice with 12 patients, palpable intraabdominal mass with 9 patients, pale stool and dark colored urine with 7 patients, fever with 6 patients, nausea and vomiting with 3 patients, lastly, anorexia with 2 patients. Abdominal pain, jaundice, and abdominal distention mostly found in patients because of the late submission to the hospital. Symptoms in older children usually stealth and intermittent, often not diagnosed properly, and later cause continuous liver damage then the patient came with liver cirrhosis and portal hypertension.2 Blood work such as bilirubin level, alpha amylase, and gamma gt could give information towards obstructive disturbance in biliary tract, such as choledochal cyst. This study stated, 5 patients with an increased total and direct bilirubin level and alkali phosphatase and gamma gt, 3 patients with increased total and direct bilirubin level and normal alkali phosphatase and gamma gt , and 3 patients with an increased alkali phosphatase and gamma gt with normal total and direct bilirubin level. The easiest supporting examination was ultrasonography to determine the type of choledochal cyst. From this study, there were 4 patients with type 1 cyst, 3 patients with type 2 cyst, and 5 patients with type 4 cyst. Other examintations for this study were MRI and abdominal CT Scan. There were 5 patients conducted Abdominal CT Scan and MRI with type 4 cyst results. Operative procedure was the definitive therapy for choledochal cyst, an intraoperative findings gave information about the type of choledochal cyst. There were 11 patients underwent explorative laparotomy surgery + cyst excision + Roux n Y hepaticojejunostomi + liver biopsy. There were 8 patients that underwent surgery, cyst size was found with >10 cm size and 3 patients with <10 cm size. Conclusions From 15 subjects with choledochal cyst in this study, there was a majority of females with age majority above 5 years old and the highest number of clinical symptom found was acute abdomen, jaundice, and intraabdominal mass. Majority of patients showed increased total and direct bilirubin, gamma gt, alkali phosphatase levels. Single types were the most found in this study. Reference 1. O’neill JA. Choledochal Cyst. Dalam: Grosfeld JL, O’Neill JA, Coran AG, FonkalsrudEW, Pediatric Surgery. Edisi ke-7. Philadelphia: Mosby Elsevier; 2006. h. 1620-31. 2. Yamataka Y, Yoshifumi Kato, Miyano T. Dalam: Ashcraft’s Pediatric Surgery. Edisi ke-5. Philadelphia: Elsevier Saunders; 2010. h. 566-73. 3. Netter F.H, ed. Atlas of Human Anatomy, 4t Edition. New York : Elsevier; 2006. p. 276, 313 4. Wing de Jong, Sjamsuhidajat. Saluran Empedu dan Hati. Buku Ajar Ilmu Bedah. Edisi ke-3. Jakarta : EGC. 2010; p 667-669. 5. Kumar mankoj, Rajagopalan B. Choledochal Cyst. Medical Journal Armed Forces India. Elsevier. India; 2012.
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